Should MASP-2 Deficiency Be Considered a Primary Immunodeficiency? Relevance of the Lectin Pathway.

Mannose-binding lectin (MBL)-associated serine protease-2 (MASP-2) is an indispensable enzyme for the activation of the lectin pathway of complement. Its deficiency is classified as a primary immunodeficiency associated to pyogenic bacterial infections, inflammatory lung disease, and autoimmunity. In Europeans, MASP-2 deficiency, due to homozygosity for c.359A > G (p.D120G), occurs in 7 to 14/10,000 individuals. We analyzed the presence of the p.D120G mutation in adults (increasing the sample size of our previous studies) and children. Different groups of patients (1495 adults hospitalized with community-acquired pneumonia, 186 adults with systemic lupus erythematosus, 103 pediatric patients with invasive pneumococcal disease) and control individuals (1119 healthy adult volunteers, 520 adult patients without history of relevant infectious diseases, and a pediatric control group of 311 individuals) were studied. Besides our previously reported MASP-2-deficient healthy adults, we found a new p.D120G homozygous individual from the pediatric control group. We also reviewed p.D120G homozygous individuals reported so far: a total of eleven patients with a highly heterogeneous range of disorders and nine healthy controls (including our four MASP-2-deficient individuals) have been identified by chance in association studies. Individuals with complete deficiencies of several pattern recognition molecules of the lectin pathway (MBL, collectin-10 and collectin-11, and ficolin-3) as well as of MASP-1 and MASP-3 have also been reviewed. Cumulative evidence suggests that MASP-2, and even other components of the LP, are largely redundant in human defenses and that individuals with MASP-2 deficiency do not seem to be particularly prone to infectious or autoimmune diseases.

[Analysis of the current situation and outlook of thoracic surgery]. / Análisis de la situación actual y previsión de futuro de la especialidad de cirugía torácica.

OBJECTIVE: The objective of this descriptive study was to analyze the current situation and forecast the future requirements for specialists in thoracic surgery, taking into account the number of doctors entering and those possibly leaving this specialty. MATERIAL AND METHODS: The data for this study were taken from the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) (n=304), Thoracic Surgeons' Club (n=122), and the Spanish Council of Medical Associations (n=225). We also took into account the current number of resident surgeons (n=84). Other specialists were included who are not recorded in these databases but who are known to be practicing (n=10). The total number of practicing specialists obtained was 211. RESULTS: There are currently 52 working thoracic surgery departments and the highest number of practicing specialists was recorded in Madrid (n=44), Catalonia (n=33), and Andalusia (n=33). The forecast number of retirements (at age 65 years) and incorporations of new specialists means that there will be a surplus of 57 thoracic surgeons in the next 5 years. CONCLUSIONS: Thoracic surgery needs to limit the intake of new trainee specialists for at least the next 5 years.

[Indicators of severity in chest trauma]. / Indicadores de gravedad en los traumatismos torácicos.

OBJECTIVE: We undertook a review of patients with chest trauma attended between January 1992 and June 2005 in order to establish severity criteria in these cases. PATIENTS AND METHODS: During the study period, 1,772 cases (1,346 [76%] males) were treated, with ages ranging from 7 to 98 years (mean, 46.4 years). The Revised Trauma Score (RTS) was calculated and the following variables were also studied as potential indicators of severity: age, extent of the injury, number of rib fractures, presence of lung contusion, hemothorax, cardiorespiratory repercussions, and need for mechanical ventilation. RESULTS: At the time of admission, 84.4% of patients presented only symptoms related to the injury, with no general repercussions, and 66.7% had an RTS of 12. The number of rib fractures was a reliable indicator of severity, as was the presence of multiple injuries, lung contusion, need for mechanical ventilation, and cardiorespiratory repercussions. Neither age nor presence of hemothorax was found to be an indicator of severity. Pleural drainage was performed in 756 cases and was effective in 670 (88.6%). CONCLUSIONS: There are a number of indicators of severity in chest trauma, related more closely to the type and repercussions of the trauma than to the age of the patient. There is a high incidence of fluid or gas accumulation in the pleural space, though this can be easily managed by pleural drainage, which constitutes the main therapeutic procedure in chest trauma.

Type of resection and prognosis in lung cancer. Experience of a multicentre study.

OBJECTIVE: Analysis of prognosis of the different types of resections for lung cancer defined by the Bronchogenic Carcinoma Cooperative Group of the Spanish Society of Pneumology and Thoracic Surgery (GCCB-S). METHODS: From October 1993 to September 1997, 2994 patients with bronchogenic carcinoma who underwent thoracotomy were prospectively recruited by the GCCB-S. Prior to recruitment, the GCCB-S had defined two types of non-resectional operations (diagnostic and exploratory thoracotomies) and three types of resections (complete-CR-: free resection margins, mediastinal nodal dissection, no extracapsular nodal involvement, no involvement of most distant removed nodes; relatively incomplete-RIR-: free resection margins, no mediastinal nodal dissection, unremoved nodes, involvement of most distant removed nodes, positive pleural effusion with no pleural implants; and incomplete-IR-: positive resection margins, extracapsular nodal involvement, unremoved positive nodes, positive pleural effusion with pleural implants). For survival analyses, patients with small cell carcinoma, induction therapy, postoperative mortality, unclassified operation, or lost to follow-up were excluded. The total number of evaluable patients was 2543. RESULTS: In 1047 (97%) patients, RIR was defined because they had undergone a lesser nodal evaluation than mediastinal nodal dissection. Five-year survival and 95% confidence interval were: diagnostic thoracotomy 11% (0-30%), exploratory thoracotomy 5% (1-9%), IR 20% (14-26%), RIR 43% (39-47%), and CR 45% (41-49%). Differences between IR and CR or RIR were statistically significant (P<0.0001), but those between CR and RIR were not (P=0.18). CONCLUSIONS: CR and RIR should be combined in a single category as complete resection, because they do not discriminate prognostic differences.